AB1273 MACROPHAGE ACTIVATION SYNDROME IN RHEUMATIC DISEASE: CLINICAL CHARACTERISTICS AND PROGNOSIS OF 20 PATIENTS
Authors: S. H. Nam, S. M. Ahn, J. S. Oh, S. Hong, C. K. Lee, B. Yoo, Y. G. Kim
Journal: Annals of the Rheumatic Diseases (2022)

Background Macrophage activation syndrome (MAS) is a hyperinflammatory condition that is known to be secondary hemophagocytic lymphohistiocytosis (HLH) in patients with rheumatic disease. Objectives The aim of study was to evaluate the clinical manifestations and outcomes in patients with MAS with rheumatic disease. Methods We performed a retrospective study of 20 adult patients who were diagnosed with MAS from 2012 to 2020. MAS was classified according to the HLH-2004 criteria. Patients’ information, including clinical features, laboratory findings, and treatment regimens, was collected, and the overall survival rate was estimated by the Kaplan–Meier method. Results Twenty patients (18 women, 35.6 ± 18.3 years) who met the HLH-2004 criteria also fulfilled the 2016 EULAR/ACR/PRINTO classification criteria for MAS, and HScore was higher than 169 (median, 238.5). Fourteen patients with systemic lupus erythematosus and 6 patients with adult-onset Still’s disease were included. All patients were treated initially with corticosteroids, and 16 patients required additional immunosuppressants. The overall survival at 3 and 6 months was 75.2% and 64.3%. In survivors, renal impairment was less common (23.1% versus 42.9%, p = 0.007), the levels of AST (202.0 versus 72.0 IU/L, p = 0.006) and LDH (1144.0 versus 343.0IU/L, p = 0.001), and platelet count (90.0 versus 46.0 × 10 9 /L, p = 0.016) were higher in compared to non-survivors. Nine patients had opportunistic infections, five of whom died during admission. Conclusion The mortality of patients with MAS remains high. Renal impairment, levels of AST and LDH, and platelet count might be associated with prognosis. Table 1. Treatments and management characteristics of patients with MAS No. Age/sex Disease Disease duration (months) 1 st Treatment (corticosteroids) 2 nd Treatment 3 rd Treatment Combined infection Alive/dead 1 19/F SLE 1 1 mg/kg IVIG + PP TCZ, RTX Bacteremia Dead 2 20/M SLE 0 1 mg/kg - - - Alive 3 20/F AOSD 1 1 mg/kg VP16 - - Alive 4 22/F SLE 1 100 mg IVIG + PP - Pneumonia Dead 5 22/F AOSD 0 500 mg IVIG - - Alive 6 23/F SLE 182 1 mg/kg - - - Alive 7 23/F SLE 41 1 mg/kg - - - Alive 8 30/F SLE 146 1 mg/kg IVIG CsA - Alive 9 32/F SLE 127 1 mg/kg IVIG + PP CsA, TCZ Pneumonia Alive 10 35/F AOSD 0 1 mg/kg CsA - Viral infection Alive 11 37/F SLE 65 1 mg/kg CsA, VP16 - Bacteremia Alive 12 38/F SLE 0 1 mg/kg IVIG + PP RTX - Dead 13 40/F AOSD 0 0.5 mg/kg CsA - - Alive 14 43/F SLE 60 1 mg/kg IVIG + PP TCZ, RTX, CsA, PCP, Dead VP16, IFX Viral infection 15 49/F SLE 0 1 mg/kg CYC - Bacteremia Alive 16 51/F AOSD 0 1 mg/kg - - - Alive 17 57/F SLE 0 1 mg/kg IVIG + PP CsA, VP16 Fungal infection Dead 18 61/F SLE 2 1 mg/kg IVIG + PP TCZ - Dead 19 68/F SLE 2 1 mg/kg IVIG + PP CsA Fungal infection Alive 20 70/M AOSD 0 1 mg/kg IVIG + PP CsA, VP16 Fungal infection Dead SLE: Systemic lupus erythematosus, IVIG: Intravenous immunoglobulin, PP: Plasmapheresis, TCZ: Tocilizumab, RTX: Rituximab, AOSD: Adult-onset still’s disease, VP16: Etoposide, PCP: Pneumocystis pneumonia, CsA: Cyclosporin, IFX: Infliximab, MCTD: Mixed connective tissue disease. Disclosure of Interests None declared